EM1511I "Non-infectious Manifestations of Common Variable Immunodeficiency: The Paradox of Autoimmunity in the Setting of Immunodeficiency" (IM GR-111315)

The purpose of this presentation is to highlight the somewhat paradoxical autoimmune, inflammatory and lymphoproliferative diseases which often occur in patients with Common Variable Immunodeficiency (CVID), their clinical presentation, and treatment. These conditions appear to be the main causes of early mortality in CVID patients, and are frequently the presenting features of the disease. The co-existence of autoimmunity with immunodeficiency is seen in other defined genetic syndromes, and some relevant examples with be discussed. This will frame a discussion on recent findings by several groups attempting to understand the genetics of CVID, a disease assumed to be polygenic in nature, and how these findings may illuminate the etiologies of autoimmune and inflammatory complications of CVID.

Learning Objectives

Understand the diagnostic criteria and prevalence of CVID and how it is distinguished from other primary antibody deficiencies.

Understand the predictors of early mortality in CVID.

Recognize the clinical and histopathologic features of the autoimmune and inflammatory complications of CVID.

Recognize that CVID should be considered on the differential diagnosis for conditions such as autoimmune cytopenias, interstitial lung diseases, inflammatory enteropathies, and nodular regenerative hyperplasia of the liver.